Intestinal malrotation

intestines1Intestinal malrotation is a developmental defect in a fetus’s intestinal tract. It occurs in roughly 1 in every 500 live births. While more boys show symptoms in the first month of life, both boys and girls are affected equally.

The problem arises at approximately the tenth week of gestation. Normally at this time, the intestine that has been developing outside the body near the base of the umbilical cord moves into the abdominal cavity.  As it moves into the abdomen it makes two rotations and is then fixed into its normal permanent position – the small bowel in the middle of the abdomen and the colon (large intestine) flanking it on both sides and across the top. This normal positioning doesn’t happen with malrotation.

With malrotation, the entire colon is positioned on the left side of the abdomen while the small intestine is on the right. The cecum (beginning of the colon) and appendix are unattached or may be incorrectly attached by bands to the duodenum (beginning of the small intestine) which can cause a blockage. Since the intestines are not properly anchored, they may twist and cut off their own blood supply, a condition known as volvulus. This is serious and can end in the loss of most of the intestine or even death.

Malrotation of the intestine usually is not evident until the intestine becomes twisted or blocked and symptoms occur. In some patients twisting and blockage do not happen and symptoms never occur, while in other patients symptoms can manifest themselves at any time. Symptoms can include greenish-yellow vomit (from bile, a digestive fluid), abdominal pain, abdominal swelling, rapid heart rate, shock, rapid breathing, or blood in a bowel movement. If you should ever see these symptoms in an infant, call the healthcare provider immediately as he will want to examine the baby. Various blood tests and diagnostic imaging (x-rays, ultrasound, etc.) may be taken to check for blockages or twisting. If they are found, surgery to correct the situation will be performed. If the blood supply to the region is compromised, it may be necessary to perform follow-up surgery to remove any permanently damaged intestine.

There usually are no long-term problems when surgery occurs before permanent damage to the intestine sets in. If large portions of damaged intestine are removed, long-term digestive problems such as the ability to absorb nutrients and liquids can be severely compromised. Nutrition may need to be obtained through long-term IV solutions.

The March of Dimes has funded nearly one million dollars in research involving intestinal malrotation and the mechanism of intestinal coiling. Hopefully, one day we will discover what causes the development to go awry and how it can be prevented in the future.

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37 Responses to “Intestinal malrotation”

  1. Shawna Says:

    Thank you March of Dimes! We need to bring more awareness to this. My son had surgery at 10 weeks to correct his malrotation.

  2. Lindsay Says:

    Shawna, I’m so glad to know your son’s malrotation was discovered and corrected. I hope he is doing well and growing like a weed. Thanks for stopping by!

  3. Laurie Watkins Says:

    There is not a lot of awareness for intestinal malrotation. It can be detected in ultrasound. There would have been a lot less heartache for my son and family if this had been diagnosed in ultrasound. Somehow this needs to be something that is looked for more tenaciously when mothers have their routine ultrasound. This can save a lot of precious time for these children that will more than likely need surgery to correct. One in five hundred births is very significant. I would love to find out about who is researching this and where specifically is he March of Dimes is funding research to ind the causes for this defect.

  4. Lindsay Says:

    Hi Laurie – I am not a scientist and cannot give clear details on our research, but I do know that we are funding research to identify genes and cellular mechanisms that cause the early embryonic gut tube to tilt to the left before forming coils. This positioning appears crucial for normal intestinal development. When it does not occur, the baby can be born with intestinal malrotation. Hopefully we will learn much from this research.

  5. Cheryl Day Says:

    So when will the March of Dimes start the awareness program on the malrotation? I am ready to help spread the word. My daughter was diagnosed at the age of 30, and suffers terribly from it. I think it continues to get worse for her. The word needs to get out NOW! I had never heard about midgut malroatation before my daughter was diagnosed, nor had anyone that I know. Even in the medical field, they do not know how to help an adult with the malrotation. I hope the March of Dimes will start walking the walk, as they are talking the talk. I will then walk right along beside them! So let’s get going, huh?

  6. Lindsay Says:

    Cheryl, we’re sorry that your daughter is facing such a hard time. Malrotation can be very difficult to live with.

    Sadly, there are roughly 5,000 different birth defects and, unfortunately, we cannot create an awareness campaign for each one. We have written the above article to draw attention to this disorder and we are funding research on the developmental biology process in order to some day understand what goes awry to cause malrotation. Hopefully from what we learn, one day we will be able to prevent this condition.

  7. Laura Says:

    Hello,
    I think that it is wonderful that you are doing this research and I thank you. We lost our four year old son to intestinal malrotation. He was a healthy boy who had no symptoms prior to the volvulous that occurred 12hrs. before. We took him to the hospital and it went undiagnosed. We were sent home with “stomach flu” and my husband found him unresponsive in the morning. It has been nearly 3.5 years and we miss him everyday and have trouble coming to terms with his death. If we had known he had malrotation we could have fixed it. Please email me if you need any further information about my son’s case. No one should have to experience this kind of preventable loss.

    Sincerely,
    Laura

  8. Lindsay Says:

    Laura, I’m so sorry for the loss of your son. I can’t imagine how devastating it was for you and your husband. Our thoughts are with you.

  9. Mary Says:

    Laura:my deepest, deepest condolences to you for your unspeakable loss. My son was diagnosed with malrotation at age 13, and after following his condition for a few years with a surgeon at a local university medical center, we made the decision to schedule him at age 16 for the surgical Ladd Procedure to alleviate the malrotation. He recovered well and we hope he can have a normal life. Although it is wonderful that March of Dimes is directing funds to research, this funding does not address the CURRENT state: 1/500 babies are born with this condition and are then sent home with parents who don’t have an awareness of this potentially fatal defect. I realize there are thousands of congenital defects, as Lindsay mentions, but this defect can result in bowel or loss of human life that could be easily prevented by ultrasound screening at birth. The occurrence of malrotation is greater than Down’s syndrome, yet we only hear about it through heartbreaking stories like yours or accidental diagnoses, as in my son’s case. I hope we see a change in the approach to this defect in the near future.

  10. Elena Barrett Says:

    Thank you march of dimes for donating money to research this birth defect my son was born with malrotation 3 years ago as well as IUGR. He was in the hospital for the first 6 months of his life, had 2 major abdominal surgeries but thanks to research funded by march of Dimes and he is now a growing energetic 3 year old! He still suffers with digestive problems and there is a need for continuing research in this area but I just wanted to say thank you for being a part of saving the life of my baby!!

  11. allison Says:

    I think its great that march of dimes has donated money for research of malrotation.I’m 37 years old and after a year and a half of pain my malrotation was discovered by accident they don’t normally check adults for this birth defect because its usually caught as a child I’ve had my ladds procedure done shortly after being diagnosed and I’m doing great 12 wks post op and before then never even heard of malrotation

  12. Liz Says:

    I am pleased to see this article. It does, however, leave the impression that this birth defect is one that manifests in infancy. While that is true, it is also true that many cases go undetected during infancy. Parents need to be aware that this particular birth defect may not create problems for many years.

    Any child with recurrent abdominal issues should be checked for malrotation, not just chalked up to having a finicky tummy. It’s a matter of life and death, as the comments show. I am an adult who was diagnosed and operated on at age 63, after having been labeled my entire adult life as someone with IBS or some sort of finicky tummy.

  13. Cindy Hughes Says:

    Hi I’m 33 years old and just found out that I was born with out left kidney and intestinal malrotation and issues with my uterus.Have low platelets and antibody that is attacking them.My appendix ruptured when I was 8 years old and they never said anything to my mom about this.I’ve been having symptoms all my live.Is there a name for this condition.Not sure if surgery is the right choice to correct this or not.

  14. Sara Says:

    Hi Cindy. We are not sure if there is a specific name for your condition. The best thing to do is to talk to your health care provider and he or she can advise you regarding your diagnosis and whether surgery may be an option. We wish you the best.

  15. Amy M Says:

    I am very excited to have read the above article and that funds are being used for research. As an adult being diagnosed late in life and now living with this debilitating condition, this brings hope. My main goal is to add “Intestinal Malrotation” to infant screening. Since this condition is 1 in 500, it’s as serious as many others infants are being screened for but those percentages are not as high as malrotation. Best of luck and again, thank you!

  16. Skyy Says:

    Hello I am a 24 year old woman who had a malrotation of my intestines when I was a little baby in 1991… it took a while for them to figure out what was really wrong with me, until my “father” got stationed in Hawaii a Hawaii hospital found out what was wrong with me after they wrongly accused my mother of neglect… they tried to say she wasn’t feeding me… but anyways I have always wondered what exactly left this HUGE scar on my tummy that I have had to grow to “like” over the years…. but here lately I have been undergoing done awful changes with my body…. like my bowel movements hurt reality bad that’s if I can go… I keep getting this horrible itchy rash on my hands and feet I’m very moody my tummy hurts all the time and I seem too be gaining weight for no apparent reason…. but I didn’t know if anyone could help me or guide me.., see I have no one to talk to about my “issues” but I thought maybe something could be wrong “in there” scared and am just looking for help…

    **BtW: I do have two beautiful sons ages 6 1-2 yrs old and 4 1-2 yrs old… I was told I was not to be able to have no kids ever.. so I just thought I might give all who are doubting anything please don’t doubt miracles do happen
    Eve though my miracles were true blessings oldest being 9 weeks early weighing at 3lbs 11oz and my youngest being 8 weeks early weighing 4lbs even… so tiny they were.. my oldest still being very small.. but so am I ( 5’3 at 105lbs) and that me putting on weight… but my boys are very healthy…. I’m nervous and babbling if anyone has any answers or questions please let me know asap…
    Sincerely, Skyylina

  17. Kathy Says:

    Hi Skyy. I just read your story. It easily could have been written by my 24 yr old daughter who was born with malrotation. My daughter at 3 weeks began exhibiting extreme pain, blood in her stools and bilious vomiting. I was also told by the doctor in the ER it was my fault because I was not feeding her incorrectly, or that she had contacted ” the flu”. My gut feeling was that she was gravely ill. Her primary physician refused to refer me to a specialist when he admitted he had no idea what was wrong with her. To make a long story a little shorter, after 5 Dr’s and many tears, my 4 month old baby had surgery #1 to correct the malrotation. At 1 yr and 4 months, she had surgery #2 to remove adhesions and had to be airlifted to Mayo. At age 6, surgery #3 for more adhesions. At 22, surgery #4 while pregnant to remove more adhesions. And surgery #5, August 2015. Her duodenum had folded over on itself. She always has bowel issues, nausea, vomiting and pain. I’m constantly searching for answers for her. We have been lucky thou. I am so thankful for the wonderful Dr’s we have had at the Mayo clinic. I hope someday they can find the cause of this birth defect. It’s been a tuff road for us but so thankful to have her. Good luck to you!

  18. Tara Says:

    Hello my name is Tara and I wanted to share about our son Ryan we lost him just 3 months ago. He was only 6 weeks old. He was a healthy baby. He had malrotation of the intestines. He showed no signs at all. On July 24 it started he cried for an hour which was unusual. He did spit up but no sign of green. We didn’t know what was wrong. He was a little gassy which usually happened everyday. Well anyway the bad started we went through our hospital to flown to St. John’s in Springfield, Illinois where they did surgery some of his intestines were dead they cut some out. Then went to St. Louis children’s hospital in Missouri his kidneys quit working before. They did what they could it was to late. All his intestines were gone. It was the worst day in our lives. He passed away in my arms in July 25 at 5:30. He was the most sweetest baby boy. I am asking hopefully soon that this will be prevented of what to look for it would save a child and the pain that us parents go through.

    Thank you from the Hancock family

  19. phil Says:

    how long was the recovery time for malrotation surgery

  20. sam Says:

    how long is malrotation surgery and whats the recovery time

  21. gemma Says:

    My beautiful baby boy sonny passed away at 4 days with volvulus just 3 weeks ago! I feel that we were let down by or hospital. When he was born he was a health boy weighing 10lbs 1.5oz, for 24 he would not feed and just slept! For a big baby we thought this was odd but were told he was fin! Before my partner left that evening sonny vomited blood yet again nurse told us it was ok! That night the nurses still tried to force cup feed him, his stomach was like a rock, i cried with concern! Finally 36 hours after birth he was brought to neo united but the didnt know what was wrong, 12 hours later he was transferred to crumlin childrens hospital where the brought him for surgery unfortunately his bowel was very much twisted! Two days later he went for another surgery to see if the bowel was getting blood….we were then meet with the devastating news that nothing could be done for our baby and we had to say our goodbyes. Im so angry that he was left to go so far and was not giving the chance of life! Ive so many questions but no answers that i need to grieve! This cannot happen to another family!

  22. Karla Says:

    Hi, my name is Karla, i am a mother of two :), my sweet angel Nicolas went to heaven at age 4, after a few hours of abdominal pain, the results came 2 days later with his autopsy report, he had intestinal malrotation and volvulus occurred while we were on our vacations at Davis, CA. Finding out the way we did, was so hard and complicated, as you all talked about, we’ve never heard of that birth defect before, we had 4 amazing years with him, with no signs of this disease, sometimes it seems unreal. After his death, they also found out he had another intestinal problems, due to this malformation. We never knew :(. Right now we are waiting for baby number two, a little girl. We’ve had all kinds of exams we’ve been recommended, nevertheless, Drs keep telling us there is no 100% positive exam that can assure us, this baby will not come with the same condition. We have met every kind of doctors, and some of them are not that familiar with this malformation. We think is very important to start making conscious to other people about this, and to have the correct examination in fetus or newborns about this fatal condition, and that doctors take more serius about stomach aches. With this new baby we have lots of questions and insecurity, we are trying to face about this disease. If any of you need more information about my son’s case, or if i could help you in anyway, please let me know.

    Love

    Karla

  23. Lhassa Says:

    I need to figure out how to get Laura’s e-mail or websight.she said we could get ahold of her if we had questions,I think my grandson may have intestinal malrotation,Thank you so much

  24. Jessica Says:

    Hello, I just recently found out my 18 year old daughter has malrotation. She also has Down Syndrome. She has always had issues with going to the restroom and was put on myralax when she was about 4 years old to try and help with this problem. It helps but she still has pain when she goes to the restroom. I am not sure if I should insist that the doctor do surgery or not. Any help would be appreciated.

  25. Tasha Says:

    Hi, currently in the ER with my daughter. Been dealing with some issues with my 6 week old baby girl. They are not wanting to do an ultrasound but instead are wanting to do an endoscopy to look. Then I’m being told if she doesn’t throw up a large green amount again that they will send us home. If they could even think it’s possible for her to have this defect how on earth would they feel comfortable just sending us home? At 4 weeks they believed she was showing signs of pyloric stenosis and then said it’s just reflux and sent us home with Zantac…. I’m so stressed and worried and they make me feel like I’m crazy

  26. Grace Says:

    My daughter was born with congenital malrotation and had the Ladd’s procedure at two weeks old. Her only symptoms were spitting up what seemed to be entire feedings and her stomach would look large and feel very firm. If you are concerned and feel like your ped is not listening, get a second opinion. I am so glad ours really listened to my concerns and sent us to get an upper GI.

  27. Tara Says:

    Hi Laura I am sorry for your loss. We had a son named Ryan he passed away at 6 weeks old. He was a healthy too we didn’t even know anything was wrong. I agree with you I wish there was a way to prevent this from happening to any other baby. He passed away July 25, 2015. We miss him everyday.

  28. Tania Says:

    My Daughter is 3 years old and was diagnosed with malroation at 5 weeks; which thank God my Husband and I pished her pediatrician to perform an upper gi. Unfortunately, we knew the symptoms so well because our oldest Son had it as well at birth. We were told that condition doesn’t happen in the same family…guess what, it did. Both kids…who are 14 and 3 years old still deal with intestinal issues and sensitive to certain foods. Please continue to research thay because I don’t know how ro help them through this part. This was good to read.

  29. Britto Says:

    Out Child was 2days old when diagnosed. He was born 23rd Sept 2016 and I am posting this comment 25th Sept 2016. As I write this he is done with first half of the surgery and the doctor would need to perform another due to the damages in the intestine. Right now he is in the ventilator. Doctors Frank answer is he wouldn’t survive and can guarantee any peace long term. I am yet to take this to his mom who has no clue about this condition. Been awake for 48 hours now and landed in this page, the research is great and I feel the pain as the others and a humble request is to do everything you can to save children. I need to leave to the hospital now to discharge her. May God Bless!

  30. Kelly Says:

    My son was born w intestinal malrotation and had surgery to correct it at 1 week old. He is now 8 wks old and struggles w every bowel movement. His surgical team and pediatrician chalk it up to normal baby problems, saying he is a healthy baby boy following the surgery. It’s hard for me to believe them when I see him going through this day after day. Is love to hear from parents whose baby had ladd procedure shortly after birth, to hear about their recovery story and long term outcome. What I’ve read here is not making me think my little man will be ok 🙁

  31. Allie Says:

    My 8 month old son had emergency surgery for malrotation when he was 4 days old. After being told he was a healthy baby in the hospital and at his first doctor appointment, I was alarmed when he was continually spitting up yellowish green his second night home. I called a nurse hotline and was reassured he was probably just spitting up colostrum, but my gut told me otherwise. I took him in the next morning only to be rushed to the children’s hospital. I am thankful all turned out okay, although he does seem to have more issues passing bowel movements than my daughter did. I wish there was more information about this given to new parents. He is my second child, yet I never knew something like this was possible.

  32. Mandy Says:

    I am 30 years old and currently 22 weeks pregnant with my first. Almost exactly one month ago I was rushed to the emergency room in the middle of the night in excruciating abdominal pain. no one knew what was wrong. Test after test, pain, vomiting, distended abdomin, finally a CT scan, showed what appeared to be a perforated bowel. I was rushed into an emergency laparotomy. Split from stern to stern, they found I wasn’t torn and leaking, but that I had been born with malrotation and it finally kinked on me. Perhaps due to the pregnancy pressure and movement of my bowels, but not for sure. my baby boy is fine! 25 staples and a nine day hospital stay. We are slowly but surly on the mends. So scary!!!! Crazy thing is my baby brother also has this same diagnosis, but his was diagnosed at 10weeks.

  33. Gladys Says:

    My son was diagnsed with malrotation at a month and a day old. He was released from the hospital after being born as a healthy baby. He showed no symptoms of being in pain or anything like that. Until one afternoon he stoped crying and began to look grayish blueish, his breathing was really low, he was lethargic, and he threw up. We rushed him to the er, they checked his vitals, temp, sugar, and everything seemed to be good. But his skin color was not normal. They did more tests and at 4:45am the next day he was getting surgery. He is currently under recovery and he is doing good. I just want to know if this is going to affect him forever? Im just so glad we brought him in on time and he is still alive here with us

  34. Kim Says:

    I had Ladds procedure when I was 5 years old. I don’t recall ever receiving any follow up and have never heard of adhesions or other post op complications. I’ve always had irregular bowel movements, but things have improved with time. I’m now almost 29 and 7 weeks pregnant. I can’t find any good resources or guides about possible pregnancy complications that I could have since having this surgery at 5 years. I already have pregnancy constipation and bloating. So far no vomiting. I will see my ob-gyn next week for the first time. How do I find out what my risks are and how to prevent further malro during the rest of the pregnancy? Any advice appreciated.

  35. Jackie Says:

    I am 54 years old and found out at 34 of my
    Malrotation but I didn’t even know that name then nor did they do anything about it except to diagnose IBS. At 39 I became pregnant and didnt even remember my malrotation but I did have a lot of trouble with swelling and gained 70 pounds plus I had high blood pressure. I had my daughter 2 weeks early because of my blood pressure and by a c-section but she was just perfect at 8 pounds. The only trouble I had was afterwards because I asked the surgeon to try to find my appendix which they were not able to find but I think by having my intestines out of my body and then put back I had a lot of trouble with constipation and my bowels but now I know why with everything floating around in there. I had laparoscopy to look for bands and my appendix removed 1.5 weeks ago. I had the same trouble with my bowels after that surgery but they found no bands which is great. I’m not a doctor but I can say that I really didn’t know any different when I was pregnant with the malrotation. I wish you the best of luck in your pregnancy and make sure you find a good doctor that understands malrotation so you both can stay on top of the situation and have a beautiful healthy baby. It took me 5 tries to get a doctor who would listen to me and understand me when I was pregnant. Make sure you take the time to find the right Dr. for you and your baby.

  36. Nicole Says:

    Hello, I am new to this my son was recently diagnosed with malrotation and has been set up to see a surgeon about this, but there are tons of questions I seem to have and my pediatrician seems to think that things will just be okay. ?’s I have are..
    Is surgery always needed for treatment of this.?
    Is my son always in pain because. He has this problem.
    Anyone who has had a 2 year old what is recovery with the surgery, and do they live healthy life styles afterwords.
    Do I always have to worry that he may die from this…

  37. Kirsty Says:

    Hi I’m a terrified mother and I carnt seem to find anything about my sons condition or tye procedure he needs.

    My sons 5 (6 in August) and has been violently sick since the day he was born. He’s finally being diagnosed with coiled and narrow bowels but what more worrying is his bowels are in the complete wrong place! If been told he’ll need open bowel surgery which would put a adult in hospital for a week. Im trying to find mums who have been through something similar and so far nobody can help me x

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